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Glioependymal/ependymal cyst is a rare central nervous system malformation defined as a subarachnoid, supratentorial, interventricular or intraspinal, sometimes intracerebral or intramedullar cyst with an internal ependymal lining, possibly surrounded by glial tissue. It may be an incidental finding or may present at different ages with clinical features depending on its size and location. It may distort adjacent brain structures and cause macrocephaly, ventriculomegaly, hydrocephalus, focal neurological signs and other signs and symptoms. In some cases, it is associated with other cerebral malformations (e.g. corpus callosum agenesis, polymicrogyria, heterotopias).
Estimated prevalence: Unknown (Unknown prevalence).
No clinical trials have been registered for glioependymal/ependymal cyst.
3 publications have been identified in PubMed for glioependymal/ependymal cyst. Research spans Review / Meta-Analysis (33%), Case Report / Case Series (33%), and Basic Science / Preclinical (33%).
Bui NE (2025). [PMID: 40041073](https://pubmed.ncbi.nlm.nih.gov/40041073/). *Surgical neurology international*. [Case Report / Case Series]
Harper SD (2025). [PMID: 40197611](https://pubmed.ncbi.nlm.nih.gov/40197611/). *Journal of neuropathology and experimental neurology*. [Basic Science / Preclinical]
Shintaku M (2025). [PMID: 40448387](https://pubmed.ncbi.nlm.nih.gov/40448387/). *Neuropathology : official journal of the Japanese Society of Neuropathology*. [Review / Meta-Analysis]
Data assembled from 3 of 12 sources · Last updated Sep 18, 2026, 7:15 PM UTC
European rare disease database
Genetic and Rare Diseases Info Center